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Background: The incidence of concurrent myasthenia gravis (MG) and neuromyelitis optica spectrum disorder (NMOSD) is higher than what chance predicts, yet it remains unclear why MG and NMOSD appear concurrently., Objective: The purpose of the present study was to examine the clinical features of the concurrence of these diseases., Methods: Clinical details were analyzed retrospectively., Results: Three (0.5%) out of 631 MG patients had confirmed (n=2) or suspected (n=1) NMOSD. Two of these patients were women. All showed early-onset MG (EOMG) that preceded NMOSD and were positive for acetylcholine receptor antibody (AChR-Ab). Two patients were tested for aquaporin 4 antibody (AQP4-Ab) and were positive. Two patients were treated with a thymectomy that preceded NMOSD. Two patients had decreased frequency of regulatory T (Treg) cells. We identified in the literature 46 patients with both MG and NMOSD. Our results of female predominance, EOMG, MG preceding NMOSD, and positive AChR-Ab are consistent with previous descriptions., Conclusions: This is the first report to examine the frequency of NMOSD in Japanese patients with MG. The reduction and/or dysfunction of Treg cells may be one cause of NMOSD development in MG., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Osteopontin (OPN) up-regulates pro-inflammatory cytokines from both T helper type 1 and T helper type 17 cell pathways. We measured plasma OPN levels in Japanese multiple sclerosis (MS) and neuromyelitis optica (NMO) patients to investigate its value as a potential biomarker of disease activity. In NMO patients, plasma OPN levels were significantly higher than those in healthy individuals, being equivalent to those in MS patients. In both NMO and MS patients, OPN levels were significantly higher during relapse compared with remission. There was also a significant positive correlation between Expanded Disability Status Scale of Kurzke scores and plasma OPN levels in both NMO and MS patients, and plasma OPN levels were significantly higher in patients with secondary progressive MS compared with those with relapsing-remitting MS. Diagnostic sensitivity and specificity of plasma OPN for MS and NMO during the relapse phase were 100% and 50%, respectively (cut-off point: 31.3ng/ml). Thus, elevated plasma OPN levels could be a potential biomarker for not only MS but also NMO. These are the first results to suggest that plasma OPN in NMO patients may be a useful marker, playing an important role in inflammation, disease activity, and disease progression, as well as MS., (© 2013.)

Introduction: Our study aim was to produce a Japanese translation of the 15-item Myasthenia Gravis Quality-of-Life Scale (MG-QOL15), assess its reliability and validity, and examine clinical factors affecting self-perceived QOL in MG., Methods: We evaluated 327 consecutive patients with MG seen at six neurological centers. All patients completed the Japanese version of the MG-QOL15 (MG-QOL15-J), the Beck Depression Inventory-second edition (BDI-II), and a generic health-related QOL questionnaire, the SF-36. Disease severity was determined according to the Myasthenia Gravis Foundation of America (MGFA) quantitative MG score and the MG composite., Results: The MG-QOL15-J exhibited adequate internal reliability, test-retest repeatability, and concurrent validity with SF-36, disease severity, and known-patient groups categorized by MGFA post-intervention status. Multivariate analysis revealed severity, dose of oral corticosteroids, and BDI-II as independent factors negatively affecting QOL., Conclusion: The MG-QOL15-J is anticipated to be a valuable clinical measure of QOL in Japanese patients with MG., (Copyright © 2012 Wiley Periodicals, Inc.)

We evaluated 30 patients with clinically definite multiple sclerosis (MS) and 8 patients with neuromyelitis optica (NMO) to investigate correlations between Th1/Th2 balance, disease activity, effects of interferon (IFN)-β treatment, and expressions of chemokine receptors CXCR3 and CCR4 on CD4+ and CD8+ T cells in peripheral blood. MS and NMO patients in the relapsing phase showed a significantly increased CD4+CXCR3+/CD4+CCR4+ ratio and CD8+CXCR3+/CD8+CCR4+ ratio compared with respective patients in the remission phase. After IFN-β treatment, the CD4+CXCR3+/CD4+CCR4+ ratio and CD8+CXCR3+/CD8+CCR4+ ratio were significantly decreased compared with the relapsing phase and slightly lower than in the remission phase. The CD8+CXCR3+/CD8+CCR4+ ratio showed a more marked change associated with disease activity than CD4+ T cells in MS and NMO patients. Moreover, in patients in the relapsing phase of NMO, the CD4+CXCR3+/CD4+CCR4+ ratio and CD8+CXCR3+/CD8+CCR4+ ratio were significantly higher than in MS patients in the relapsing phase. We confirmed marked changes in the CD8+CXCR3+/CD8+CCR4+ ratio according to disease activity and treatment of MS and NMO. Furthermore, this ratio was more strongly linked to immune and inflammatory activity in NMO patients than in MS patients, and may represent an important factor in differentiating the pathogenesis of MS and NMO., (Copyright © 2011 S. Karger AG, Basel.)